Guillain-Barre Syndrome F082

Current RMA Instruments

Changes from previous Instruments

ICD Coding

• ICD-10-AM Code: G61.0

Brief description

Guillain-Barré syndrome is an immune-mediated disorder in which the body's immune system mistakenly attacks the nerves outside the brain and spinal cord. It develops over a relatively short period and can affect muscle strength, sensation and automatic body functions such as heart rate and blood pressure. The most common form causes rapidly increasing weakness, usually affecting both sides of the body, together with reduced or absent reflexes. People may also experience numbness, tingling or other changes in sensation. Other forms of Guillain-Barré syndrome can produce different patterns of nerve involvement.

The severity varies considerably. Some people have relatively mild weakness, while others may develop severe weakness or paralysis, difficulty swallowing, or weakness of the muscles needed for breathing. Recovery usually occurs gradually over weeks to months, although some people may have ongoing weakness or other neurological symptoms.

Confirming the diagnosis

Guillain-Barré syndrome is complex and always requires diagnostic confirmation by a Neurologist. The diagnosis is based on the characteristic clinical presentation supported by appropriate neurological investigations. The clinical pattern varies according to the subtype but generally involves an acute or subacute development of motor, sensory and/or autonomic neurological dysfunction.

The diagnosis is normally confirmed by electrodiagnostic testing demonstrating features consistent with an acute peripheral neuropathy and/or cerebrospinal fluid examination demonstrating an elevated protein concentration without an elevated white cell count (albuminocytological dissociation).

Importantly, supportive investigations may be normal early in the illness, and diagnosis therefore requires interpretation in the context of the clinical presentation and evolution of neurological findings. MRI may be undertaken where required to investigate alternative diagnoses but is not generally required to confirm Guillain-Barré syndrome.

Additional diagnoses covered by SOP

• Acute inflammatory demyelinating polyneuropathy
• Acute motor axonal neuropathy
• Acute motor sensory axonal neuropathy
• Miller Fisher syndrome
• Other recognised variant forms of Guillain-Barré syndrome

Conditions excluded from SOP

• Chronic inflammatory demyelinating polyneuropathy #
• Infective polyneuropathy #
• Other peripheral neuropathies not representing a variant of Guillain-Barré syndrome *

* another SOP applies

# non-SOP condition

Clinical onset

Clinical onset refers to the earliest point at which symptoms and signs attributable to Guillain-Barré syndrome first developed. Guillain-Barré syndrome has an acute or subacute onset, with neurological symptoms progressing over a relatively short period. The initial manifestation is commonly limb weakness, sensory symptoms or a combination of these, although cranial nerve, autonomic or other neurological manifestations may predominate in some variants.

Clinical onset is based on the earliest documented or reliably reported neurological manifestation that can be attributed to the subsequently confirmed Guillain-Barré syndrome by a Neurologist. 

Clinical worsening

Guillain-Barré syndrome typically progresses during an acute phase before reaching a plateau and subsequently entering a recovery phase. During the acute illness, there may be progression of limb weakness, increasing sensory or cranial nerve involvement, autonomic instability, bulbar dysfunction or respiratory muscle weakness. Assessment by a Neurologist is recommended when evaluating whether deterioration represents the expected evolution of the acute illness, a complication, or what could be regarded as a clinical worsening.

Appropriate clinical management of this condition may include close neurological and respiratory monitoring, intravenous immunoglobulin or plasma exchange, management of autonomic complications and ventilatory support where required.